Nonsyndromic thoracic aortic aneurysm and dissection: outcomes with Marfan syndrome versus bicuspid aortic valve aneurysm

Andrew G. Sherrah, Sarah Andvik, Denise Van Der Linde, Lucy Davies, Paul G. Bannon, Ratnasari Padang, Michael P. Vallely, Michael K. Wilson, Anthony C. Keech, Richmond W. Jeremy*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

34 Citations (Scopus)


Background Genetic aortopathy (GA) underlies thoracic aortic aneurysms (TAA) in younger adults. Comparative survival and predictors of outcomes in nonsyndromic TAA (NS-TAA) are incompletely defined compared to Marfan syndrome (MFS) and bicuspid aortic valve (BAV). Objectives The study sought to compare survival and clinical outcomes for individuals with NS-TAA, MFS, and BAV. Methods From 1988 to 2014, all patients presenting with GA 16 to 60 years of age were enrolled in a prospective study of clinical outcomes. Risk factors for death and aortic dissection were identified by Cox proportional hazards modeling and a mortality risk score developed. Results Diagnosis of GA was made for 760 patients (age 36.9 ± 13.6 years, 26.8% female; NS-TAA, n = 311; MFS, n = 221; BAV, n = 228). MFS patients were younger than NS-TAA and BAV. Presentation with aortic dissection was more common for NS-TAA than MFS or BAV. The 687 patients surviving >30 days after presentation were followed for a median of 7 years. Calculated 10-year mortality was 7.8% for NS-TAA, 8.7% for MFS, and 3.5% for BAV (NS-TAA and MFS vs. BAV p <0.05). Factors associated with all-cause mortality were MFS (p = 0.04), age at presentation, and family history of dissection. Conclusions Clinical outcomes for MFS and NS-TAA are similar but worse than BAV. Independent predictors of mortality, including family history of aortic dissection and age, can be included in an Aortopathy Mortality Risk Score to predict survival. Management of NS-TAA, including surgical intervention, should be similar to that of MFS.

Original languageEnglish
Pages (from-to)618-626
Number of pages9
JournalJournal of the American College of Cardiology
Issue number6
Publication statusPublished - 16 Feb 2016
Externally publishedYes


  • aortic surgery
  • genetic aortopathy
  • mortality
  • risk


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